title: Maximal daily dose of pancreatic enzyme replacement therapy in infants with cystic fibrosis: a reconsideration.
abstract: The current recommendations for dosing of pancreatic enzyme replacement therapy (PERT) in infants with cystic fibrosis (CF) were made using a limited evidence base. The per meal recommended dose was extrapolated from dosing guidelines for older patients into a maximal daily dose for infants. We discuss why this maximal daily dose recommendation may be insufficient for young infants with CF, although the optimal dose of PERT for infants with CF remains unknown.
authors.affiliation: State University of New York at Buffalo School of Medicine and Biomedical Sciences, Department of Pediatrics, Women and Children's Hospital of Buffalo, Buffalo, NY, United States. Electronic address: dborowitz@upa.chob.edu.
authors.forename: Drucy
authors.initials: D
authors.lastname: Borowitz
authors.forename: Daniel
authors.initials: D
authors.lastname: Gelfond
authors.forename: Karen
authors.initials: K
authors.lastname: Maguiness
authors.forename: James E
authors.initials: JE
authors.lastname: Heubi
authors.forename: Bonnie
authors.initials: B
authors.lastname: Ramsey
mesh_terms: D003550:Cystic Fibrosis / Q000188:drug therapy*; D056947:Enzyme Replacement Therapy; D006801:Humans; D007223:Infant; D007227:Infant Nutritional Physiological Phenomena; D008049:Lipase / Q000008:administration & dosage* / Q000627:therapeutic use
chemical_list: D008049:Lipase
keywords: Cystic fibrosis; Infant nutrition; Pancreatic enzymes; Pancreatic insufficiency